Achalasia is a motor disorder of the esophagus characterized by ineffective peristalsis in the distal esophagus and lack of coordinated relaxation of lower esophageal sphincter (L.E.S.). Primary achalasia is most common in children and is associated with absence of ganglion cells in the esophageal myenteric plexus. It is rare in children and less than 5% of patients are under 15 years of age. The etiology is unknown, but an autoimmune inflammatory process is being implicated.

Diagnosis: Observational, cross-sectional.
Children with Achalasia may present with vomiting, regurgitation, dysphagia, recurrent pneumonia or failure to thrive. Clinical suspicion is the key to diagnosis.

The radiological features of achalasia cardia may be difficult to demonstrate in early disease. Barium swallow under fluoroscopic guidance is the initial investigation of choice. Incomplete esophageal clearing and retrograde movement of barium within the esophagus are early signs. Dilatation of the esophageal body and tapering of the barium column at the unrelaxed LES (bird-beak sign) is characteristic of well established disease.

Upper G-1 endoscopy demonstrates the dilated esophagus with a tight L.E.S. Manometry reveals elevated LES pressure or incomplete LES relaxation. Manometry is difficult to perform and interpret in young children. CT and MRI findings are non specific and are not indicated. Ultrasound and angiography are not useful in the diagnosis of Achalasia.

Treatment: Therapeutic options include medications that relax smooth muscle, pneumatic dilatation and surgical myotomy. Pharmacologic therapy; usually Nifedipine or Isosorbide has limited, often transient efficacy and may be used only in those with mild disease or those who are too sick for more aggressive therapy. The current approach is based on stretching or cutting the muscle fibres at the esophago-gastric junction by pneumatic balloon dilatation or surgical myotomy. While earlier studies reported less than 50% efficacy with balloon dilatation, more recent studies have shown it to be as effective in children as in adults. The clinical improvement after a balloon dilatation should be sustained for at least 2 years, if it can be termed successful. Balloon dilatation has the advantage of being a simple, quick and safe procedure, especially if the esophagus is markedly dilated. Heller's myotomy is the surgical procedure of choice and has a success rate of more than 80%. Laparoscopic approach is most appropriate and offers the surgeon the opportunity to perform an anti-reflux surgery as well.

Botulinum toxin (Botox) works by inhibiting the release of acetylcholine from pre-synaptic nerve terminals. The drug needs to be injected into the LES by the endoscopist. Results in adults have not given satisfactory results and there are no published trials in children.

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